Molecular weight (DA)
187kD
Immunogen
Synthesized peptide derived from human protein . at AA range: 450-530
Specificity
ABCA3 Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. The full transporter encoded by this gene may be involved in development of resistance to xenobiotics and engulfment during programmed cell death. [provided by RefSeq, Jul 2008],
Function
disease:Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921]; also called pulmonary alveolar proteinosis due to ABCA3 deficiency. Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular non-specific interstitial pneumonitis (NSIP).,domain:Multifunctional polypeptide with two homologous halves, each containing an hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.,function:Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.,similarity:Belongs to the ABC t
Protein name
ATP-binding cassette sub-family A member 3 (ABC-C transporter) (ATP-binding cassette transporter 3) (ATP-binding cassette 3)
Fields
>>ABC transporters
Human protein sequence Database
Q99758
Mouse protein sequence database
Q8R420
Rat protein sequence database
Cellular localization
Endosome, multivesicular body membrane ; Multi-pass membrane protein . Cytoplasmic vesicle membrane . Late endosome membrane . Lysosome membrane . Localized in the limiting membrane of lamellar bodies in lung alveolar type II cells (PubMed:22673903, PubMed:16959783, PubMed:24142515, PubMed:27177387, PubMed:11718719). Trafficks via the Golgi, sorting vesicles (SVs) and late endosome/multivesicular body network directly to the outer membrane of lamellar bodies in AT2 lung epithelial cells or to lysosomes and lysosomal-related organelles (LROs) in other cells where undergoes proteolytic cleveage and oligosaccharide processing from high mannose type to complex type (PubMed:24142515, PubMed:20863830, PubMed:16959783, PubMed:27177387). Oligomers formation takes place in a post-endoplasmic reticu
Tissue expression
Expressed in brain, pancreas, skeletal muscle and heart (PubMed:8706931). Highly expressed in the lung in an AT2-cell-specific manner (PubMed:11718719, PubMed:8706931). Weakly expressed in placenta, kidney and liver (PubMed:8706931). Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma (PubMed:8706931).
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